Clinical features of autopsy-confirmed multiple system atrophy in the Mayo Clinic Florida brain bank

Shunsuke Koga, William P. Cheshire, Philip W. Tipton, Erika D. Driver-Dunckley, Zbigniew K. Wszolek, Ryan J. Uitti, Neill R. Graff-Radford, Jay A. van Gerpen, Dennis W. Dickson

Research output: Contribution to journalArticlepeer-review


Background: Multiple system atrophy (MSA) presents with various combinations of autonomic dysfunction, parkinsonism, and cerebellar ataxia. Although clinical diagnostic criteria have been widely used, the sensitivity and specificity are suboptimal. This study aims to provide evidence supporting the revision of the current diagnostic criteria for MSA. Methods: Medical records of 171 patients with autopsy-confirmed MSA in the Mayo Clinic brain bank were reviewed with regard to their clinical features and diagnoses. Pathologic features, including concomitant pathologies (i.e., Alzheimer-related and Lewy-related pathologies), were also assessed. Results: The cohort included 133 MSA-parkinsonian type, 36 MSA-cerebellar type, and 2 unclassified MSA patients who did not show significant motor symptoms. Twenty-three patients (13%) were not clinically diagnosed with MSA, but instead with progressive supranuclear palsy, Parkinson's disease (PD), PD with dementia (PDD), or dementia with Lewy bodies (DLB). Three patients with PDD and DLB also had concomitant Lewy body pathology. Six patients had late-onset MSA, with an age of onset greater than 75 years. Erectile dysfunction was frequent in male patients (60/63; 95%) in all age ranges. REM sleep behavior disorder (RBD) was present in 82 patients (48%) and was the initial symptom in 13 patients. Cognitive impairment was present in 60 patients (35%), but was an initial symptom in only two patients. Conclusions: Our findings support the conclusion that late-onset presentation should not exclude MSA. The findings of this large autopsy-based cohort provides valuable insights for improving clinical criteria for MSA.

Original languageEnglish (US)
Pages (from-to)155-161
Number of pages7
JournalParkinsonism and Related Disorders
StatePublished - Aug 2021


  • Cognitive impairment
  • Dementia with lewy bodies
  • Diagnostic accuracy
  • Erectile dysfunction
  • Late-onset MSA
  • Multiple system atrophy
  • Neuropathology
  • Parkinson's disease
  • Progressive supranuclear palsy
  • REM sleep Behavior disorder

ASJC Scopus subject areas

  • Neurology
  • Geriatrics and Gerontology
  • Clinical Neurology


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